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Objective:To explore the effect of intermittent angle traction on the supine position combined with acupoint application on the changes of cervical radiculopathy related biological parameters.Methods:Randomized controlled trial. A total of 100 patients with radiculopathy cervical spondylosis treated in the Second Affiliated Hospital of Heilongjiang University of Traditional Chinese Medicine from February 2019 to February 2021 were selected and randomly divided into observation group ( n=50) and control group ( n=50). Patients in the control group were given conventional treatment with western medicine, and patients in the observation group were given acupoint application combined with supine intermittent angle traction combined with treatment. Both groups were treated for 3 months. The Japanese Orthopaedic Association Spinal Cord Function (JOA), Cervical Disability Index (NDI), Pain Visual Analogue Score (VAS) and the changes in intervertebral foramen volume of two groups were compared before and after treatment. The difference in clinical efficacy between the two groups after treatment was observed. We took a thin spiral CT film of the patient's cervical spine, established a three-dimensional finite element model of the cervical spine, and compared the differences in related indicators between the two groups before and after treatment. Results:The total effective rate was 94.0% (47/50) in the observation group and 70.0% (35/50) in the control group, and there was a significant difference between the two groups ( χ2=9.76, P=0.002). After treatment, the JOA score in the observation group was significantly higher than that of the control group ( t=6.23, P<0.01), the NDI score, VAS score were significantly lower than those in the control group ( t values were 5.17,9.13, P<0.01), the intervertebral foraminal volume [(8.45±1.27)mm 3vs. (7.18±1.38)mm 3, t=4.79] was significantly higher than that of the control group ( P<0.01), and C 4-7 cervical vertebra flexion [(7.15±0.87)° vs.(5.64±0.78)°, t=9.14], retroflexion [(8.53±0.73)° vs. (7.15±0.68)°, t=9.78], sidebend [(6.57±0.71)° vs. (5.28±0.67)°, t=9.34], rotated [(7.89±0.52)° vs. (6.54±0.48)°, t=13.49] were significantly higher than those in the control group ( P<0.01); C 4-7 disc flexion [(1.41±0.09) kPa vs. (2.01±0.12) kPa, t=28.28], retroflexion [(1.54±0.07) kPa vs. (2.01±0.08) kPa, t=31.26], sidebend [(1.24±0.07) kPa vs. (1.89±0.13) kPa, t=31.13], rotated [(1.23±0.06)kPa vs. (1.85±0.11)kPa, t=34.99] were significantly lower than those in the control group ( P<0.01). Conclusion:The supine intermittent Angle traction combined with acupoint application in the treatment of cervical spondylotic radiculopathy can relieve the neck pain, improve the neck function, restore the physiological curvature of the cervical spine, and promote the recovery of cervical biomechanics.
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@#Resection is one of the most important treatments for esophageal squamous cell carcinoma, and routine postoperative follow-up is an effective method for early detection and treatment of recurrent metastases, which can improve patients' quality of life and prognosis. This consensus aims to provide a reference for colleagues responsible for postoperative follow-up of esophageal squamous cell carcinoma patients in China, and further improve the standardization of the diagnosis and treatment of esophageal squamous cell carcinoma.
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The current primary treatment of cervical esophageal cancer is still the definitive chemoradiotherapy. Salvage esophagectomy is recommended for patients who have failed the therapy. Cervical esophageal cancer is closely adjacent to the surrounding important organs, and the boundaries of regional tissues become more blurred after radiotherapy, which pose a great challenge to surgical treatment. Successful completion of this operation requires a comprehensive grasp of various surgical techniques such as total larynx, pharynx, esophagus, partial tracheal resection and respective reconstruction. The author introduces several core issues of this operation, such as tumor resection, digestive tract reconstruction, airway reconstruction, and lymph node dissection, in order to provide references for domestic colleagues, improve the success rate of surgery, and reduce the occurrence of fatal complications.
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In the era of multidisciplinary treatment of esophageal cancer, especially the development of immunotherapy, the aim of organ-sparing treatment is to improve quality of life without reducing survival. After neoadjuvant therapy, the common efficacy evaluation methods are endoscopic biopsy and imaging examination, which may help patients with complete response obtaining organ-sparing treatment. Liquid biopsy technology represented by circulating tumor cells is a novel evaluation method, which will be used in multimodality evaluation to guide clinical decision making in the future. For organ-sparing treatment, it should consider the period of surgical window and optimization of combined treatment (chemotherapy regimens and radiation doses, etc.) to achieve a higher pathologic complete response rate. In the era of immunotherapy, it is necessary to explore effectively predictive biomarkers to identify patients who are suitable for organ-sparing treatment, and individualize the best combination therapy for different patients. At the same time, strengthening the intensity of consolidation therapy is also an important role for immunotherapy in organ-sparing treatment. Active surveillance is required to detect recurrence and implement salvage therapy for patients who received organ-sparing treatment. It is expected that the organ-sparing treatment of esophageal cancer could be developed under the new era of immunotherapy.
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Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a highly stimulated and defective inflammatory response caused by genetic inheritance or acquired immune regulation abnormalities.Lymphoma-associated hemophagocytic syndrome (LAHS) is a malignancy-associated HLH secondary to lymphoma, with a high clinical misdiagnosis rate and fatality rate and poor prognosis.In this article, the pathogenesis, diagnosis and treatment of LAHS in children were reviewed, in order to increase clinician′s understanding of the disease.
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Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a highly stimulated and defective inflammatory response caused by genetic inheritance or acquired immune regulation abnormalities.Lymphoma-associated hemophagocytic syndrome (LAHS) is a malignancy-associated HLH secondary to lymphoma, with a high clinical misdiagnosis rate and fatality rate and poor prognosis.In this article, the pathogenesis, diagnosis and treatment of LAHS in children were reviewed, in order to increase clinician′s understanding of the disease.
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Objective: In this study, black tea and Citrus maxima (BT-CM), yellow tea and C. maxima (YT-CM), green tea and C. maxima (GT-CM) as subjects, the active ingredient content and antioxidant activity of three tea and C. maxima (T-CM) were analyzed. The effects of three T-CMs on apoptosis of liver cells in vitro and its mechanism were further explored. Methods: National standard method and HPLC were used for active ingredient analysis. MTT, cell flow cytometry and Western blot were used to analyze the effects of three T-CMs on cell proliferation, apoptosis, and its underlying molecular mechanism. Results: The content of tea polyphenols, free amino acids, ratio of polyphenols and amino acids, ester catechins, non-ester catechins and caffeine in YT-CM and GT-CM was significantly higher than that of BT-CM. The in vitro antioxidant capacity of YT-CM and GT-CM was also significantly stronger than that of BT-CM. Three T-CMs had the effects of inhibiting proliferation, arresting cell cycle and inducing apoptosis in HepG2 and Bel7402 cells, especially YT-CM and GT-CM. Western blot analysis showed three T-CMs activated PI3K/AKT/mTOR signaling pathway and regulated the expression levels of apoptosis-related proteins Bax, Bcl-2 and Caspase-3/9. YT-CM and GT-CM had better ability to change the signal pathway than BT-CM. Conclusion: In short, T-CMs, which combined different degrees of fermentation tea with C. maxima, were rich in nutrients and biologically active substances. T-CMs, especially YT-CM and GT-CM, are healthy drinks that help to prevent and treat liver cancer.
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Cell migration is defined as the directional movement of cells toward a specific chemical concentration gradient, which plays a crucial role in embryo development, wound healing and tumor metastasis. However, current research methods showed low flux and are only suitable for single-factor assessment, and it was difficult to comprehensively consider the effects of other parameters such as different concentration gradients on cell migration behavior. In this paper, a four-channel microfluidic chip was designed. Its characteristics were as follows: it relied on laminar flow and diffusion mechanisms to establish and maintain a concentration gradient; it was suitable for observation of cell migration in different concentration gradient environment under a single microscope field; four cell isolation zones (20 μm width) were integrated into the microfluidic device to calibrate the initial cell position, which ensured the accuracy of the experimental results. In particular, we used COMSOL Multiphysics software to simulate the structure of the chip, which demonstrated the necessity of designing S-shaped microchannel and horizontal pressure balance channel to maintain concentration gradient. Finally, neutrophils were incubated with advanced glycation end products (AGEs, 0, 0.2, 0.5, 1.0 μmol·L -1), which were closely related to diabetes mellitus and its complications. The migration behavior of incubated neutrophils was studied in the 100 nmol·L -1 of chemokine (N-formylmethionyl-leucyl-phenyl-alanine) concentration gradient. The results prove the reliability and practicability of the microfluidic chip.
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Cell Movement , Chemotaxis , Equipment Design , Lab-On-A-Chip Devices , Microfluidic Analytical Techniques , Microfluidics , Neutrophils , Reproducibility of ResultsABSTRACT
Neuroblastoma-amplified sequence (NBAS) is a highly conserved gene firstly found in neuroblastoma, targeting on the human chromosome 2 p24.3 and encodes a protein that is a subunit of the Syntaxin 18 complex. The functions of NBAS include the involvement in transport of Golgi-to-Endoplasmic Reticulum retrograde and degradation of nonsense-mediated mRNA. NBAS gene is widely expressed in more than 30 tissues, suggesting that it may play an important role in human body. In 2010 and 2015, NBAS was identified successively as the pathogenic gene of SOPH syndrome and fever-related liver failure respectively. Recent studies shows that NBAS gene mutations can involve immune system and cause immune deficiency. In this paper, we review the biological function of NBAS, NBAS gene mutation-related diseases and their pathogenesis in recent years.
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Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time.
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Objective:Explore the " project-talent-base" combining international science and technology cooperation management model and mechanism through summarizing the practical experience of a tertiary pediatric hospital in Beijing in recent years, to provide reference for strengthening the hospital′s international science and technology cooperation.Methods:Main problems existing in the management of international science and technology cooperation in domestic medical institutions are analyzed, and management methods of international science and technology cooperation adopted by the hospital are classified through policy sorting, literature research, summarization and classification.Results:The hospital has established an efficient and high-quality international science and technology cooperation management model mainly through four aspects: improving the international cooperation management system, enriching international cooperation and exchange projects, building different types of international science and technology cooperation platforms, and strengthening personnel training.Conclusions:Under the background of the globalization of science and technology and the accelerating pace of scientific research in China, improving the hospital′s international science and technology cooperation management model will effectively promote the introduction of international advanced technologies and concepts, and enhance the overall science and technology innovation capabilities and discipline construction.
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Objective:To explore the hospital-institute integration mode based on project cooperation.Methods:Focusing on the management innovation of clinical and basic scientific research cooperation mode, the method of hospital-institute integration mode based on project cooperation and the key points in practice were discussed, to develop better understandings of the construction and development such mode.Results:Several key points of developing hospital-institute integration mode based on project cooperation were proposed. Main measurements adopted including the integration of scientific research and clinic service, comprehensive construction of management concept, scientific research capacity, teaching organization and service concept, cultivation of high-level research talents, construction of research departments, according to which formed the main structure of the hospital. Furthermore, it is important to change the development mode in time to promote the healthy development of the hospital.Conclusions:It is a new direction of the clinical and basic research cooperation innovation to encourage the hospital-institute integration mode based on project cooperation. It is also an effective strategy to promote the improvement of diagnosis and treatment level under the new medical reform situation.
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Natural killer group 2 member D(NKG2D) is an immune receptor expressed by NK cells that recognizes the human major histocompatibility complex class I polypetide-related chain(MIC) A/B on the cell surface.The interaction between NKG2D and MICA/B plays an important role in the immunosurveillance of viruses infection and cancers.In this article, we review the research progress of the MICB/NKG2D signaling pathway in immune escape including three parts: down-regulation of membrane-bound MICB, increase of secretory MICB, and polymorphism of MICB genes.
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Objective The experiment project was designed to explore the variation of NaI (Tl) gamma spectrometer channels with environmental temperature. 60Co and 152Eu were used to verify the reliability of the correction methods. Methods Two correction methods were applicated, which were curve fitting correction method and known measurement peak correction method. Results The experimental results showed that temperature changes had an effect on NaI (Tl) measured spectra peak. The relative peak will shift to the right at 5℃ by 9.6%, and to the left at 60℃ by 16%, with the reference temperature set at 25℃. The two methods are based on the channel change due to temperature changes, and they could effectively correct the temperature peak-drift. Conclusion In order to make the measured spectrum information accurate and reliable in field monitoring, it is suggested to monitor the environmental temperature so as to correct the measured data.
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Objective:To explore the effect of self-made protective clothing in tracheal intubation for the patients with respiratory infectious diseases.Methods:Self-made protective clothing were made by adult model plastic raincoat with sleeve lets and goggles. A prospective randomized controlled study was conducted. Patients with severe respiratory infectious diseases who needed tracheal intubation admitted to the department of intensive medicine of the Third Affiliated Hospital of Gansu University of Chinese Medicine from January 1st 2018 to March 31st 2020 were enrolled. According to the random number table method, they were divided into two groups. The control group was wearing standard protective clothing, while the test group was wearing self-made protective clothing for endotracheal intubation. The wearing time, infection rate of operators and costs of protective clothing were compared between the two groups. The patients were sprayed with trypanosome blue diluent before tracheal intubation, and the whole body of the operator was photographed with fluorescence before wearing self-made protective clothing and after doing tracheal intubation to take off the self-made protective clothing, in order to evaluate the permeability resistance of self-made protective clothing.Results:A total of 86 patients were enrolled. There were 46 cases in the test group, included 28 cases of influenza A (H1N1) virus infection, 11 cases of influenza B virus infection and 7 cases of adenovirus infection. There were 40 cases in the control group, included 15 cases of H1N1 virus infection, 10 cases of influenza B virus infection, 10 cases of adenovirus infection and 5 cases of unknown pathogen. There was no significant difference in respiratory etiology between the two groups ( χ2 = 3.789, P = 0.435). The wearing protective clothing time of the control group was 11.6 times than that of the test group (minutes: 22.23±1.45 vs. 1.86±0.24, χ2 = 19.023, P < 0.001). The cost of standard protective clothing was 12.5 times than that of self-made protective clothing (Yuan/set: 500 vs. 40). Fluorescent photography showed that the whole body of the operator was not stained after tracheal intubation, indicating that the protective clothing had good anti permeability and achieved the protective effect. There was no operator infection in the test group and the control group. Conclusion:Self-made protective clothing has short wearing time, low cost and equivalent isolation effect compared with standard protective clothing, which is worthy of clinical promotion.
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Objective:To explore the effect of locking compressing plates (LCP) internal fixation under arthroscopy combined with Zhuyu Tongluo method in the treatment of distal radius fractures (DRF) and its effects on joint function, hemorheology, serum basic fibroblast growth factor (bFGF) and bone morphogenetic protein 2 (BMP-2).Methods:A total of 78 patients with DRF admitted to the Second Hospital of Tangshan City from June 2017 to January 2020 were selected and grouped according to the random number table method, with 39 cases in each group. The control group was treated with LCP internal fixation under arthroscopy, and the study group was treated with Zhuyu Tongluo method on the basis of the control group. The recovery, hemorheology (plasma viscosity, whole blood viscosity high cut, whole blood low cut, hematocrit), serum bFGF, BMP-2 levels, before and after the operation were compared between the two groups. They were followed up for 6 months and the range of motion of the wrist joints before and after the operation and the excellent and good rate of wrist joint function between the two groups were compared.Results:The swelling subsidence time, pain disappearance time, ecchymosis disappearance time, and fracture imaging healing time in the study group were shorter than those in the control group: (10.37 ± 3.13) d vs. (14.62 ± 3.38) d, (12.39 ± 2.97) d vs. (17.14 ± 4.02) d, (19.15 ± 2.35) d vs. (24.36 ± 3.27) d, (68.21 ± 7.12) d vs. (80.07 ± 8.24) d, and the differences were statistically significant ( P<0.05). The plasma viscosity, whole blood viscosity high-cut, whole blood low-cut, and hematocrit in the study group were lower than those in the control group at 1 and 2 weeks after operation ( P<0.05). The levels of serum bFGF and BMP-2 in the study group were higher than those in the control group at 1 and 2 weeks after operation: at 1 week after operation: (356.27 ± 46.29) μg/L vs.(236.51 ± 37.42) μg/L, (614.28 ± 61.47) μg/L vs. (487.39 ± 48.24) μg/L; at 2 weeks after operation:(502.07 ± 113.21) μg/L vs. (381.59 ± 82.75) μg/L, (910.77 ± 97.20) μg/L vs. (731.35 ± 92.13) μg/L, and the differences were statistically significant ( P<0.05). The palm flexion, back extension, radial deviation, and ulnar deviation in the study group were better than those in the control group at 6 months after operation ( P<0.05). The excellent and good rate of wrist joint function in the study group was higher than that in the control group at 6 months after operation: 89.5%(34/38) vs. 70.3%(26/37), and the difference was statistically significant ( P<0.05). Conclusions:LCP internal fixation under arthroscopy combined with Zhuyu Tongluo method in the treatment of patients with DRF can increase the expression of serum bFGF and BMP-2, reduce inflammation, improve blood rheology, promote fracture healing, and improve wrist range of motion and wrist function.
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Objective:To understand the clinical characteristics and prognosis of Langerhans cell histiocytosis (LCH) with skin-limited lesion.Methods:A retrospective analysis was performed on clinical characteristics and prognosis of 16 skin-limited LCH patients, out of 578 LCH patients who were hospitalized in Beijing Children′s Hospital during December 2013 to June 2018.Results:A total of 16 skin-limited LCH cases, accounted for 2.7% of all 578 cases, were included.Among which, sex ratio (male vs.female) was 1.28∶1.00.Median ages of skin eruption occurrence and of diagnosis of the disease were 3.5 months (3 days to 2 years and 5 months) and 6 months (2 months 14 days to 2 years and 8 months) in this group.Among the 16 cases, seborrheic dermatitis-like lesions(11 cases, 68.7%) was the most common, and the trunk was most frequently involved[75.0% (12 cases)]. Serine/threonine protein kinase gene V600E [ BRAF (p.V600E)] mutation was detected in pathological specimens from 10 skin-limi-ted cases, with 9 cases being positive.Plasma samples from 5 positive cases were further detected for BRAF (p.V600E) mutation, and 4 positive results were gained.Of all 16 patients, 11 cases (68.7%) were treated.Remission were achieved in 3-6 months from treatment start in patients treated whether according to the Histiocyte Society′s LCH-2009 protocol for 25 weeks(6 cases, 37.5%), or with topical mometasonefuroate for 3 months (3 cases, 18.8%). Two patients(12.5%) with solitary cutaneous lesions underwent excision biopsy (one face and one prepuce) and were considered to be in remission immediately after surgery.None of these patients suffered from the recurrence of the disease.The remaining 5 patients (31.3%) with skin-limited LCH were just evaluated regularly, and achieved remission in 3-6 months of commencing observation.Among these untreated patients, 1 with consistently positive BRAF (p.V600E) mutation in plasma had bone involvement in the 24 th month of assessment, and was then treated based on the Histiocyte Society′s LCH-2009 Protocol.No clinical or imageological evidence supporting disease progression was found on this patient.Median follow-up period was 32.8 months (2.9-63.9 months). Except one patient, none of the rest cases had active disease till follow-up ended.Two-year event free survival(EFS) of this research was (92.3± 7.4)%.There was no significant difference between EFS of treated group and that of observation group( χ2=1.250, P=0.264). Conclusions:Skin-limited LCH often occurs in infants and newborns, with strong heterogeneity in clinical manifestations, laboratory indicators, and pathogenesis.Seborrheic dermatitis-like lesions were the most common cutaneous type.The prognosis of the patients is excellent despite progressing into multisystem involvement can be seen in a few patients.
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OBJECTIVE：To investigate the correlation between CYP2C19 gene polymorphisms and antiplatelet reactivity of clopidogrel in Xinjiang Uygur patients with acute coronary syndrome （ACS）. METHODS ：Totally 90 Uygur patients with ACS who were admitted to the cardiovascular department of Xinjiang Kashi Second People ’s Hospital from Jan. 2018 to Jan. 2019 were selected as the study subjects. They were given anti-platelet therapy of asprin+clopidogrel ，and received the treatment continuously for one year after discharge. The platelet aggregation inhibition （DPAI）rate of the patients were determined ，and the response to clopidogrel was evaluated. PCR-fluorescence probe method was used to detect genotype of CYP2C19*2 and * 3，and PCR-direct sequencing method was used to detect genotype of * 17. Multivariate Logistic regression analysis was adopted to investigate the correlation of gene and non-gene factors with DPAI of patients. RESULTS ：Among 90 patients，there were 10 patients with clopidogrel resistance （CR）and 80 patients with non-CR. There were 58，28 and 4 patients with CYP2C19*2 G/G，G/A，A/A genotype，respectively；there were 88 and 2 patients with CYP2C19*3 G/G，G/A genotype ，respectively；there were 64 and 26 patients with CYP2C19*17 C/C，C/T genotype ，respectively；the genotype frequency of each genotype was consistent with Hardy-Weinberg equilibrium （P＞0.05）. After treatment ，DAPIs of patients with CYP2C19*2 G/A，A/A genotype were decreased significantly，while those of the patients with A/A genotype were significantly lower than patients with G/A genotype patients （P＜ 0.05）. DAPIs of patients with CYP2C19*17 C/T genotype were increased significantly ，compared with C/C genotype 2016D01C087） patients （P＜0.05）. There was no statistical significance in @fudan.edu.cn DAPIs between CYP2C19*3 G/A and A/A genotype patients （P＞0.05）. Multivariate Logistic regression analysis showed 85775264@qq.com that CYP2C19 gene pol ymorphism was independently related to DPAI in Xinjiang Uygur patients with ACS [OR ＝2.314，95％CI（1.569，3.144），P＝0.009]，while age ，gender，smoking and other non-gene factors were not related to DPAI （P＞0.05）. CONCLUSIONS ：CYP2C19 gene polymorphism is associated with antiplatelet reactivity of clopidogrel in Xinjiang Uygur patients with ACS. * 2 wild type patients may have higher DPAI ，while * 17 wild type patients may have lower DPAI.
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Objective:To evaluate the prognostic value of Epstein-Barr virus (EBV)-DNA level in plasma and whole blood in treatment of children with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH).Methods:Clinical data of 66 children with EBV-HLH, who were admitted to the Hematology and Oncology Center of Beijing Children′s Hospital, Capital Medical University from January 2016 to December 2017 were retrospectively reviewed and analyzed.The data included the dynamic changes of the EBV-DNA level in plasma (P-EBV-DNA) and whole blood (W-EBV-DNA) at the time of admission, 2 and 4 weeks after treatment.P-EBV-DNA was divided into the positive group and the negative group according to the copy number of EBV-DNA, and W-EBV-DNA was divided into the high and the low level group by the receiver operating characteristic curve(ROC); the incidence of poor prognosis was compared between different groups by Chi- Square test; the event-free survival (EFS)was evaluated by the Log- Rank test to identify its prognostic significance. Results:The analysis showed that both P-EBV-DNA and W-EBV-DNA at admission could not be associated with a poor outcome; P-EBV-DNA (≥500 copies/mL) or W-EBV-DNA [>(5.04-5.09)×10 5 copies/mL]after 2 and 4 weeks of treatment could be a good marker of a poor outcome and progression-free survival ( P<0.001). Besides, central nervous system (CNS) involvement ( P=0.025), sever leukopenia(WBC≤3×10 9/L, P=0.031), neutropenia (ANC ≤0.5×10 9/L, P=0.041), albumin reduction (≤26 g/L, P=0.012) and hemoglobin decrease (≤90 g/L, P=0.023) at diagnosis are also associa-ted with worse outcomes.In multivariate analysis, only P-EBV-DNA at 4 th week and CNS involvement were indepen-dent prognostic factors ( HR=7.139, P=0.032 and HR=6.455, P=0.042, respectively). The prognostic value of W-EBV-DNA at different time points and P-EBV-DNA after 2 weeks of treatment had a lower prognostic value. Conclusions:The P-EBV-DNA level after 4 weeks of treatment is a promising risk indicator for early diagnosis of disease and early recognition of poor prognosis in EBV-HLH children, so it provides the guidance for optimal treatment.
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Hemophagocytic lymphohistiocytosis(HLH)is a critical hematological disorder characterized by immune dysfunction with multiple organs and systems involved.HLH secondary to rheumatic disease is also named as macrophage activation syndrome(MAS). In children, MAS is often covered by primary diseases so that it is hard to be discovered early, associated with critical condition and the difficulty of diagnosis and treatment.In this review, we summarize the advances on pathogenesis, diagnosis and treatment of MAS to improve the understanding of MAS and contribute to clinical diagnosis and treatment.